The group is responsible for developing and publishing consensus guidelines, based on all available medical and scientific evidence, for the treatment of primary and recurrent. Wed like to understand how you use our websites in order to improve them. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Chordoma in the lateral medullary cistern in a patient. License allowing third parties to download articles and share them with others as long as they credit the authors and the publisher, but without. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. Earlier studies suggested a more aggressive behavior compared with classical chordoma, 39 yet most recent studies consider the chondroid variant as simply a chordoma. The most common tumors in the clival region are chordomas, with an overall incidence of 0. We report a case of congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus. Chordoma of the clivus is a rare invasive and destructive tumor. Providing care for a chordoma patient is complex and time. Radiation oncologychordoma wikibooks, open books for an.
Metastatic disease to the clivus mimicking clival chordomas. The initial treatment for a clival chordoma is maximal safe surgical removal. The evening brings together his passion for jazz music and the passionate restructuring and insolvency community of which he is a part. For more inferior lesions, we have modified the transoraltranspharyngeal approach by. We developed the following chordoma educational materials to help you make informed medical decisions and get the best care possible for yourself or your loved one. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body, such as the lungs. The clival chordoma and chondrosarcoma are the most common of these tumors. Visual complications of proton beam therapy for clival chordoma. Case report within the first days after birth, a female newborn presented with torticollis, muscular hypotonia, and acro cyanosis. Visual complications of proton beam therapy for clival. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. A chordoma is a rare type of cancerous tumor that can occur anywhere along the spine, from the base of the skull to the tailbone. Chordomas of the clivus are skull base tumors which arise from stem cells of the chorda dorsalis in front of the basilar artery.
They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body. A phase ii study has been completed using 800 mgday imatinib. A vertebral extra dural chordoma at c5, possibly deriving. Harris center for chordoma care is dedicated to providing unsurpassed compassionate care for your patient while advancing the art and science of evaluating, diagnosing, treating, curing, and supporting the patient with chordoma. Sustained response of a clivus chordoma to erlotinib after.
Chordoma of the clivus a bone in the middle of the head is the most common location for the development of a chordoma, which is a primary bone cancer that develops from remnants of embryonic notochord in the skullbase head and spine. Among 50 patients, 70% had stable disease, and 64% obtained a clinical benefit. Transmaxillarytransnasal approach to the anterior clivus. Thirtyfive percent of chordomas occur in the sphenooccipital region.
However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. Kilpatrick, in practical surgical neuropathology, 2010. Large retroclival prepontine mass infiltrating the posterior aspect of the clivus and compressing the brain stem posteriorly. A 41yearold male presented with an incidental clival lesion presumed to be a chordoma. The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the notochord regresses during. Purely ectopic pituitary adenomas are exceedingly rare. At ct, intracranial chordoma typically appears as a centrally located, wellcircumscribed, expansile softtissue mass that arises from the clivus with associated extensive lytic bone destruction. More detailed information about the symptoms, causes, and treatments of chordoma is available below symptoms of chordoma. In the t1 weighted sagittal images they represent distensions of the clivus with secondary displacement of the pituitary gland. About 300 people are diagnosed with chordoma in the united states each. Chordoma is a kind of cancer that grows in the bones of your skull and spine. Endoscopic endonasal resection of a clival chordoma with massive. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge the diagnostic acumen of theclinician. Conventional chemotherapy is not effective for locally recurrent or metastatic chordoma.
The previously mentioned metastatic chordoma with cutaneous lesions was a case of a clivus chordoma arising in. Chordoma is a rare, slowgrowing malignant tumor arising from notochordal remnants. At first, radiological imaging did not reveal a tumoral condition, though intraoperative exploration and tissue histology revealed a chordoma which eroded her clivus and had a transdural extension. Major differential diagnoses of the chordoma in the clivus are the other central. Activation of pdgfra and pdgfrb was documented in chordoma 11, 12, leading to the clinical evaluation of imatinib, a pdgf inhibitor 4, 5.
The transseptaltranssphenoidal approach is appropriate for biopsy or for subtotal removal of small midline lesions of the upper retrosellar clivus only. Clival chordoma is a rar e intracranial neoplasm located in the clivus with bony extension and destruc tion. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. The classic appearance of intracranial chordoma on ct is a welldefined expansile soft tissue mass that arises from the clivus with associated lytic bone change. Clival ectopic pituitary adenoma mimicking a chordoma. Pdf endoscopic endonasal approach for clival chordomas. The classical radiologic feature of the clivus chordoma is a destructive softtissue mass with calcification due to sequestra. Roentgen examination will often show osteolysis of the clivus.
Magnetic resonance imaging revealed a small tumor at the clivus. Typically the mass projects posteriorly at midline, indenting the pons. Nonchordomatous lesions of the clivus jama network. Common symptoms reported by people with clival chordoma.
Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Eight of the patients were men and four were women. The most common locations for a chordoma are at the triangular bone near the base of the spine sacrum, the coccyx, and the clivus, which is a bone in the base of the skull. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. A 60year old caucasian woman presented with meningitis secondary to cerebrospinal fluid rhinorrhoea. Clival chordomas are a rare type of cancer with low metastatic potential and primary metastasize to the lung or bones. The sphenoid sinus and upper clivus are then exposed for tumor removal. The following flow chart illustrates the possible options for a skull base the bones at the bottom part of the skull that separate the brain from other structures. Calcification within the tumor occurs more frequently than reported in the past. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. Chondroid chordoma is a variant of chordoma recognized by the who, but the significance of this variant is debated. The treatment of 21 patients with a diagnosis of chordoma seen over a 20year period is described. Expert recommendations for the diagnosis and treatment of. By 1999, optic neuropathy had developed in 12 of the 274.
To download or request hard copies of these materials, please use the form at the end of the page. Patients with a clival metastasis typically have diplopia due to an. Eighteen months later, he sought treatment for a right 6th nerve palsy. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. These sites are the ends of the neuraxis and the vertebral bodies. Chordoma educational materials download or request a. Characteristics and patterns of metastatic disease from. In the t1 weighted sagittal images they represent distensions of the clivus with secondary displacement of the. Itposes differentialdiagnostic problems fortheradiologist aswell. Cranial ch is a very rare entity in the pediatric population, since it represents less than 1% of intra cranial malignancies and 5% of all chs. Surgical approaches to clivus chordoma are discussed. The mean age at first operation was 51 years range, 10 to 80.
The gold standard treatment for chordomas of the mobile spine and sacrum is enbloc excision with wide margins and. A 19yearold man had been treated for a chordoma of the clivus with surgical removal using the suprainfratentorial approach, followed by postoperative radiosurgery. Sections show a lobulated tumor with cells arranged in cords and sheets, present in a predominantly myxoid and focally sclerotic stroma. Dec 28, 2017 chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Skull base surgery for clival chordoma treatment pacific. The approaches described in this article have been used in an attempt to minimize morbidity while maximizing exposure. The chordoma foundation clinical trials program supports welljustified clinical trials designed to determine whether, and to what extent, promising new therapies can improve the lives of chordoma patients.
The low incidence of treatmentrelated morbidity from proton beam therapy for clivus chordoma is felt to be within acceptable limits. It is hypointense in t1, markedly hyperintense in t2 and shows minimal enhancement post contrast. Chordoma symptoms, diagnosis, treatments and causes. Clival lesions are rare and represent a wide range of different benign and malignant pathologies. Pdf chordomas of the clivus are aggressive lesions which arise from the remnants of the primitive notochord and pose unique diagnostic and management. It is a malignant bone tumour 14% of all primary bone tumors.
The clivus is a part of the posterior skull base where the basal occipital bone joins the sphenoid bone. Chords for chordoma is stevens way of giving back to other canadians with chordoma and their circles of love. We report an autopsy case of clival chordoma in a 61yearold woman, who underwent surgery 6 times for. Guidelines for skull base isolated recurrence chordoma. Chordoma nord national organization for rare disorders. Treatment guidelines for locally recurrent chordoma. Transcranial approaches to clival chordomas provide a circuitous route to the site of origin of the tumor often involving extensive bone drilling and. When a chordoma arises in the nasopharynx, ethmoid air cells, and nasal cavity at the most rostral end of the neural axis, it is often excluded from the differential altogether. In rare cases, the sphenoid sinus can pneumatize the whole. On november 9, 2017, some of north americas top jazz musicians will gather at the toronto centre for the arts in toronto for a musical celebration.
Given their midline location, the great majority of clival chordomas and chondrosarcomas are best removed via an endoscopic endonasal approach however, some extensive andor laterally placed chordomas may require different skull base approaches. Characteristics and patterns of metastatic disease from chordoma. We present a case of a clival chordoma that had severe brainstem. The abducens nerve tracks along the clivus before it enters the cavernous sinus and ultimately the superior orbital fissure okudo and anusim, 2016. These tumors seldom arise from the cervical or thoracic vertebrae. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between 1971 and 1989. Apr 30, 2003 the low incidence of treatmentrelated morbidity from proton beam therapy for clivus chordoma is felt to be within acceptable limits. Endoscopic endonasal transclival resection of chordomas. Features are characteristic of a chordoma which was subsequently confirmed on biopsy.
This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Deriving from the embryonic remnants of the notochord, chordomas are a rare type of cancer. Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Gathering along with them will be canadas restructuring and insolvency community taking the opportunity to honour one of their own. The treatment recommendations you find here were developed by the chordoma global consensus group a multidisciplinary, international group of over 60 doctors who have extensive experience caring for chordoma patients. The clival region is the second most common location, accounting for 3035% of cases 2,3. Chordoma of the clivus areport of four cases byhans f.
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